12例肝淀粉样变性患者临床特征分析  

Clinical features of patients with hepatic amyloidosis

作　　者：余思邈 王亚超 朱云[1] 王立福[1] 杜宁[1] 孙永强[1] 许文涛[1] 王丽苹[1] 景婧[1] 张帆[1] 王睿林[1] YU Simiao;WANG Yachao;ZHU Yun;et al(Diagnosis ＆ Treatment and Research Center of Liver Disease by Integrated Traditional Chinese and Western Medicine, 302 Hospital of PLA, Beijing 100039, China)

机构地区：[1]解放军第三〇二医院中西医结合肝病诊疗与研究中心,北京100039

出　　处：《临床肝胆病杂志》2018年第4期836-839,共4页Journal of Clinical Hepatology

基　　金：国家自然科学基金面上项目(81673806)

摘　　要：目的分析肝淀粉样变性患者的临床特点。方法收集2011年1月-2016年12月于解放军第三〇二医院肝病科住院治疗的12例确诊为肝淀粉样变性患者的临床资料,观察患者的一般资料、临床症状、体征、实验室检查结果、影像学特点、病理特点等。结果 12例肝淀粉样变性患者中,男11例(91.7%),女1例(8.3%);年龄40~67岁,平均(53.0±8.5)岁,其中AA型肝淀粉样变性2例(16.7%),AL型肝淀粉样变性10例(83.3%)。临床症状主要表现为乏力7例(58.3%),纳差5例(41.7%),肝区不适疼痛9例(75.0%),蛋白尿8例(66.7%)。影像学检查肝硬化12例(100%),肝大12例(100%),脾大8例(66.7%)。实验室检查Alb水平下降8例(66.7%),肝功能异常11例(91.7%),TBil水平升高7例(58.3%),GGT水平升高12例(100%),ALP水平升高11例(91.7%)。结论肝淀粉样变性多发于中年男性,临床出现肝硬化、肝大、蛋白尿、肝功能异常、ALP及GGT水平升高时,需高度警惕肝淀粉样变性可能,进行活组织病理检查可确诊。目前该病无有效的特异性治疗方法,但早期诊断、早期干预,及时处理相关并发症,可对患者预后起到一定的改善作用。Objective To investigate the clinical features of patients with hepatic amyloidosis.Methods A retrospective analysis was performed for the clinical data of 12 patients with hepatic amyloidosis who were hospitalized in Department of Hepatology,302 Hospital of PLA,from January 2011 to December 2016,including general information,clinical symptoms,signs,laboratory findings,imaging features,and pathological features.Results Of all 12 patients,there were 11 male patients（91.7%） and 1 female patient（8.3%）,aged 40-67 years（mean 53.0 ± 8.5 years）,and 2（16.7%） had AA hepatic amyloidosis and 10（83.3%） had AL hepatic amyloidosis.Major clinical symptoms included weakness in 7 patients（58.3%）,poor appetite in 5 patients（41.7%）,hepatic discomfort and pain in 9 patients（75.0%）,and proteinuria in 8 patients（66.7%）.Imaging examination showed liver cirrhosis in 12 patients（100%）,hepatomegaly in12 patients（100%）,and splenomegaly in 8 patients（66.7%）.Laboratory examination showed reduced albumin in 8 patients（66.7%）,abnormal liver function in 11 patients（91.7%）,increased total bilirubin in 7 patients（58.3%）,increased gamma-glutamyl transpeptidase（GGT） in 12 patients（100%）,and increased alkaline phosphatase（ALP） in 11 patients（91.7%）.Conclusion Hepatic amyloidosis mainly occurs in middle-aged men.The possibility of hepatic amyloidosis should be considered in case of liver cirrhosis,hepatomegaly,proteinuria,abnormal liver function,and increased ALP and GGT in clinical practice.Liver biopsy helps to make a definite diagnosis.There are still no effective therapies for this disease,but early diagnosis and intervention and timely treatment of complications can improve patients’ prognosis.

关 键 词：肝疾病 淀粉样变性 疾病特征 

分 类 号：R575.5[医药卫生—消化系统]